Farhi D C, Shikes R H, Murari P J, Silverberg S G
Cancer. 1983 Oct 15;52(8):1516-25. doi: 10.1002/1097-0142(19831015)52:8<1516::aid-cncr2820520831>3.0.co;2-n.
The clinical and pathologic features of 23 cases of hepatocellular carcinoma occurring in patients younger than age 35 years (mean age, 17.4 years) were analyzed. Ten of these (43%) were the fibrolamellar oncocytic variant (FLO), characterized by large polygonal neoplastic hepatocytes and lamellar bundles of collagen. The remainder (non-FLO) showed the usual wide range of gross and histologic patterns typical of hepatocellular carcinoma in older age groups. Overall, hepatocellular carcinoma was more common in females than in males. The FLO variant was characterized by a longer duration of symptoms prior to diagnosis, increased frequency of resectability of the tumor, and infrequency of mitoses. Of particular importance is the fact that 5 of 10 patients with the FLO variant are alive and clinically free of disease 1 1/2 to 8 years postoperatively, while none of the 13 patients with non-FLO hepatocellular carcinoma is alive and free of disease. There was no significant difference between the two groups in mean age at diagnosis, presence of single versus multiple hepatic tumors, vascular invasion, or tumor necrosis. Although cirrhosis was present in three non-FLO patients and none of the FLO patients, the difference was not statistically significant. The prognosis of hepatocellular carcinoma in young patients does not appear to differ from that in older patients, with the exception of the fibrolamellar oncocytic variant, a variant which is common in younger patients.
分析了23例年龄小于35岁(平均年龄17.4岁)的肝细胞癌患者的临床和病理特征。其中10例(43%)为纤维板层嗜酸性细胞变异型(FLO),其特征为大的多边形肿瘤性肝细胞和板层状胶原束。其余病例(非FLO)表现出老年组肝细胞癌常见的广泛大体和组织学模式。总体而言,肝细胞癌在女性中比在男性中更常见。FLO变异型的特征是诊断前症状持续时间较长、肿瘤可切除性频率增加以及有丝分裂频率较低。特别重要的是,10例FLO变异型患者中有5例在术后1.5至8年仍存活且临床上无疾病,而13例非FLO肝细胞癌患者中无一例存活且无疾病。两组在诊断时的平均年龄、单发与多发肝肿瘤的存在、血管侵犯或肿瘤坏死方面无显著差异。虽然3例非FLO患者存在肝硬化,而FLO患者中无一例存在,但差异无统计学意义。年轻患者肝细胞癌的预后似乎与老年患者无异,纤维板层嗜酸性细胞变异型除外,该变异型在年轻患者中常见。